Idiopathic Juxtafoveal Telangiectasis Research Articles

PERIPHERAL RETINAL ANGIOGRAPHIC FINDINGS IN MACULAR TELANGIECTASIS TYPE 2.

To evaluate the retinal periphery in patients with idiopathic juxtafoveal telangiectasis or macular telangiectasis Type 2 (MacTel2), using widefield fluorescein angiography. Single-center, retrospective, observational case series of 50 eyes of 50 patients with MacTel2 and 50 eyes of 50 age-matched controls. Thirty-seven eyes in the MacTel2 group (74%) showed peripheral capillary nonperfusion or dropout, compared with 37 eyes in the control group (74%, P = 1.0). Morphologically, the MacTel2 group trended toward having a higher proportion of pruning-type capillary dropout (44%) compared with controls (28%), but this was not statistically significant (P = 0.12). Patients with MacTel2 had a higher incidence of microaneurysms compared with controls (MacTel2 56%; controls 42%; P = 0.048), independent of age or systemic risk factors. There was no difference in the incidence of venous-venous shunts (MacTel2 10%; controls 10%; P = 1.0), arteriovenous shunts (MacTel2 14%; controls 18%; P = 0.60), venous tortuosity (MacTel2 60%; controls 66%; P = 0.58), or arterial tortuosity (MacTel2 54%; controls 68%; P = 0.20), which was mild in most cases. We note a high incidence of peripheral vascular and retinal findings in both patients with MacTel2 and age-matched controls, using widefield fluorescein angiography. Patients with MacTel2 had significantly more microaneurysms, independent of age or other systemic risk factors.

SHORT-TERM SAFETY OF 2 MG INTRAVITREAL ZIV-AFLIBERCEPT.

To evaluate the safety of single intravitreal 2 mg ziv-aflibercept (0.08 mL) injections for the treatment of choroidal neovascular membranes (CNVM). Eyes with choroidal neovascular membranes because of several conditions each received single intravitreal injections of 2 mg ziv-aflibercept (0.08 mL). Comprehensive ophthalmic examinations and detailed systemic evaluations were performed at baseline and Days 1, 7, and 30 after injections. Standard electroretinography was performed at baseline and Day 30. Primary outcome measures consisted of safety assessments (signs of clinical toxicity and electroretinographic abnormalities). Secondary outcome measures included changes in best-corrected visual acuity (BCVA) and central subfield thickness (CST) of the macula. Twenty-one eyes of 20 patients (12 males) received injections. Etiologies responsible for the choroidal neovascular membranes included age-related macular degeneration (14), polypoidal choroidal vasculopathy (PCV) (3), myopia (2), and idiopathic juxtafoveal telangiectasia (2). None of the patients complained of worsening vision or pain after the intravitreal injections and no intraocular inflammation was seen. No significant changes in the electroretinographic b/a ratio from baseline to 1 month were measured (scotopic: P = 0.89; photopic: P = 0.13) and mean intraocular pressures were unchanged (14.2 ± 3.6 vs. 13.7 ± 3.0 mmHg; P = 0.62). Mean best-corrected visual acuity did not change significantly from baseline to 1 month (0.66 ± 0.37 logMAR [Snellen equivalent: 20/100] vs. 0.61 ± 0.35 logMAR [Snellen equivalent: 20/80]; P = 0.72) but significant improvements in central subfield thickness were seen (343 ± 177 vs. 210 ± 133 μm; P = 0.01). Single intravitreal injections of 2 mg ziv-aflibercept (0.08 mL) appear to be safe through 1 month.

Idiopathic Juxtafoveal Telangiectasia

Idiopathic Juxtafoveal Telangiectasia (IJT), also called Idiopathic Macular Telangiectasia, is a group of disorders with varying etiologies that share the common finding of telangiectatic vessels in the juxtafoveal region of the macula. IJT is classically divided into three groups, each with clinically dis-tinct findings and differing etiologies. Type I typically occurs unilaterally, primarily affects males, and is thought to be congenital. Vision loss usually results from macular edema. The most common type, II, normally presents bilaterally, has no sex predilection, and is thought to be acquired. Vision loss occurs not from edema, but from retinal atrophy. The last type, III, is extremely rare and is usually found in association with coexisting systemic or neurological disorders. Vision loss most likely results from retinal isch-emia. This paper will describe classifications, clinical findings, etiologies, and treatment options of IJT along with patient case figures.

Efficacy of Intravitreal Bevacizumab in Treatment of Proliferative Type 2 Idiopathic Juxtafoveal Telangiectasia.

Objectives:To evaluate the effectiveness of intravitreal bevacizumab (IVB) in patients with subretinal neovascularization secondary to type 2 juxtafoveal telangiectasia.Materials and Methods:Ten eyes of 10 patients were included in this retrospective study. All cases were treated with IVB (1.25 mg bevacizumab). Visual acuity and slit-lamp anterior and posterior segment examinations were performed at each visit. Central macular thickness (CMT) and intraretinal/subretinal fluid were evaluated via spectral domain optical coherence tomography (OCT). Loss of a line in visual acuity chart and presence of fluid on OCT were defined as criteria for repeated treatment.Results:The mean age of patients was 66.0±7.0 years (56-75). The mean follow-up time was 54.7±16.0 month (24-72). The mean BCVA was 0.62±0.35 (0.00-1.00) logMAR at baseline and 0.54±0.35 (0.00-1.00) logMAR at final exam (p=0.03). The mean CMT was 251±25.4 µm at baseline and 239±39.3 µm at final exam (p=0.01). Patients received an average of 1.7±1.0 IVB injections during follow-up. At baseline, all cases had intraretinal/subretinal fluid. There was no fluid at final examination of all cases.Conclusion:IVB treatment may be effective in the treatment of subretinal neovascularization secondary to type 2 juxtafoveal telangiectasia.

Intravitreal bevacizumab in the treatment of idiopathic juxtafoveal telangiectasis type I

Clinical caseWe report a case of a 42-year-old male with a macular edema due to idiopathic juxtafoveal retinal telangiectasis type I, treated with 3 sequential injections of intravitreal bevacizumab (1.25mg in 0.05ml). Anatomical improvements were observed after one year of follow up. DiscussionThere is currently no general consensus regarding the treatment of unilateral idiopathic juxtafoveal telangiectasis. The therapeutic options are, grid laser photocoagulation, intravitreal triamcinolone, verteporfin photodynamic therapy, or anti-VEGF. Visual acuity and anatomical improvements were observed in this case after intravitreal bevacizumab. Thus, intravitreal bevacizumab seems to be effective to treat macular edema in idiopathic juxtafoveal telangiectasis type I.

Bevacizumab intravítreo como tratamiento de las telangiectasias yuxtafoveales idiopáticas tipo i

Clinical caseWe report a case of a 42 year-old male with a macular edema due to idiopathic juxtafoveal retinal telangiectasis type i, treated with 3 sequential injections of intravitreal bevacizumab (1.25mg in 0.05ml). Anatomical improvements were observed after one year of follow up. DiscussionThere is currently no general consensus regarding the treatment of unilateral idiopathic juxtafoveal telangiectasis. The therapeutic options are, grid laser photocoagulation, intravitreal triamcinolone, verteporfin photodynamic therapy, or anti-VEGF. Visual acuity and anatomical improvements were observed in this case after intravitreal bevacizumab. Thus, intravitreal bevacizumab seems to be effective to treat macular edema in idiopathic juxtafoveal telangiectasis type i.

MORPHOLOGIC CHARACTERISTICS OF IDIOPATHIC JUXTAFOVEAL TELANGIECTASIA USING SPECTRAL-DOMAIN AND POLARIZATION-SENSITIVE OPTICAL COHERENCE TOMOGRAPHY

Idiopathic juxtafoveal telangiectasia (IJT) is characteristically associated with pigmentary changes. Polarization-sensitive spectral-domain optical coherence tomography (PS-SD-OCT) enables imaging of the retinal pigment epithelium (RPE) and similar melanin-containing structures based on specific polarization properties. This study examined IJT with the latest-generation SD-OCT and PS-SD-OCT, identifying pathophysiologically relevant characteristics of the retinal layers and RPE. Twenty-two eyes of 12 patients with IJT were examined by PS-SD-OCT, with special focus on RPE detection and segmentation. Furthermore, SD-OCT technology (Cirrus, Spectralis, and 3D-OCT-1000) was applied. Characteristics of the retinal layers and RPE were evaluated. A classification system based on OCT characteristics of IJT was suggested. Polarization-sensitive spectral-domain optical coherence tomography together with SD-OCT identified characteristic patterns of IJT, used to classify eyes into three distinct groups. Group 1 (5 eyes) revealed discrete alterations in the inner retinal layers; group 2 (12 eyes) showed irregularities of the junction between the inner and outer photoreceptor segments with outer retinal atrophy but an intact RPE. Group 3 (5 eyes) revealed RPE irregularities and loss in addition to intraretinal alterations and photoreceptor abnormalities. This study described characteristic morphologic changes in IJT based on PS-SD-OCT and SD-OCT. Morphologic changes were classified, possibly leading to an OCT-based grading scheme. The intensity images of SD-OCT verified intraretinal and photoreceptor irregularities in great detail, whereas PS-SD-OCT additionally showed RPE alterations.

Clinical Features of Idiopathic Juxtafoveal Telangiectasis in Koreans

PurposeTo describe the clinical characteristics of idiopathic juxtafoveal telangiectasis (IJT) in Koreans.MethodsMedical records of 16 patients with IJT were analyzed during the period from 1997 to 2009. Diagnosis was based on biomicrosopic and fluorescein angiographic findings and the group was determined according to the Gass and Blodi classification.ResultsWe analyzed eight patients in group 1A (50%), two in group 1B (12.5%), and six in group 2A (37.5%). Diverse treatment modalities, such as macular laser photocoagulation, photodynamic therapy, intravitreal antiangiogenic agent, and steroid injection, were applied for macular edema in nine eyes; however, only two eyes showed visual improvement.ConclusionsIn this case series, group 1A was the most common. For macular edema related to IJT, current treatment strategies had no consistent effect.

A New Vascular Pattern for Idiopathic Juxtafoveal Telangiectasia Revealed by the Retinal Function Imager

The Retinal Function Imager (RFI) (Optical Imaging, Rehovot, Israel) is a novel method for assessing retinal blood flow characteristics and demonstrating the dynamics of fine retinal vasculature. The authors report a new pattern of retinal blood flow in five patients with idiopathic juxtafoveal telangiectasia (IJT) type 2. Retinal circulation is imaged using the RFI as a series of high-speed stroboscopically captured fundus photographs. Proprietary software aligns the images, allowing serial subtraction, and creates short video loops that show movement of red blood cells in the small and medium size retinal blood vessels. Ten eyes of five patients with IJT type 2 were examined. In all cases, the RFI demonstrated a common pattern of centripetal vascular flow in the region of telangiectasia. This pattern is unique in its configuration of vascular flow into the focus of the area of telangiectasia. This pattern could not be appreciated on fluorescein angiography. The RFI can detect the specific vascular pattern of the retinal blood flow in eyes of patients with IJT. The ability to characterize such blood flow patterns may aid in identification of ambiguous cases of IJT when the diagnosis is not straightforward.

Resolution of Macular Edema in Idiopathic Juxtafoveal Telangiectasis Using PDT

A 57-year-old woman was treated by photodynamic therapy for macular edema due to idiopathic juxtafoveal telangiectasis (presumed type 1A) without subretinal neovascularization. Initial visual acuity of the treated eye was 20/200 and it improved to 20/40 by 3 months after the photodynamic therapy session. Visual acuity remained stable 32 months after the treatment. Color photographs and fundus fluorescein angiography before and after photodynamic therapy revealed regression of hemorrhages, exudates, and fluorescein leakage. Photodynamic therapy has long-term benefits for the patient with idiopathic juxtafoveal telangiectasis, presumed type 1A, because it can improve visual acuity and macular edema.

Intravitreal bevacizumab (Avastin) for subretinal neovascularization secondary to type 2A idiopathic juxtafoveal telangiectasia

To evaluate the efficacy and safety of intravitreal bevacizumab (Avastin) in the treatment of subretinal neovascularization (SRNV) secondary to type 2A idiopathic juxtafoveal telangiectasia (IJT). Intravitreal bevacizumab (1.25 mg/0.05 ml) was injected as primary treatment into six eyes of six patients with SRNV due to IJT in this nonrandomized, interventional case series. The best-corrected visual acuity (BCVA) was measured and the optical coherence tomography (OCT) and fundus fluorescein angiography (FFA) findings were examined before and after treatment. The patients were followed up for 3-6 months. Pre-injection BCVA measured 20/400 to 20/120 (mean 20/200). After a mean follow-up of 4.2 months, post-injection BCVA measured 20/200 to 20/50 (mean 20/100). At last visit BCVA improved two or more lines in five eyes (83%) and remained the same in one eye (17%). The mean central foveal thickness improved from 263 microm (range, 165 to 393 microm) to 201 microm (range, 126 to 351 microm), representing an average reduction of 62 mum. Only one eye received more than one (2) bevacizumab injections. No significant complications were observed. In this small series, intravitreal bevacizumab appears to be a safe and effective treatment for SRNV secondary to type 2A IJT.

Injeção intravítrea de triancinolona no tratamento da telangiectasia retiniana justafoveolar idiopática

Case report of one idiopathic juxtafoveal telangiectasis (IJT) 1A patient whose right eye was treated with a 4 mg intravitreal triamcinolone acetonide injection. The outcome was evaluated by visual acuity and optic coherence tomography. The visual acuity and the caliper retinal thickness before triamcinolone injection were respectively 20/100 and 569 microm, and 20/60 and 371 microm after three weeks and 20/100 and 614 microm after six week of follow-up. The stabilization of the vascular wall due to the intravitreal triamcinolone injection leads to a transitory improvement in vision and reduction in macular edema in the TJI 1A eyes. No permanent help by the photocoagulation could be shown.

Intravitreal steroids in the management of macular oedema

The use of intravitreal corticosteroids in the management of macular oedema has recently gained widespread acceptance. New long-acting steroid preparations and methods of delivery have facilitated the use of these new modalities. This review describes the various types of macular oedema for which this therapeutic option is used and the results.

Optical Coherence Tomography of Idiopathic Juxtafoveolar Telangiectasia

To document optical coherence tomography (OCT) findings in a series of eyes with group 2A idiopathic juxtafoveal telangiectasia. This study is a retrospective review of patient charts, OCT, fundus photography, and fluorescein angiography involving 23 eyes (12 patients). Mean retinal thickness in 9 macular areas was calculated and compared to previously published measurements from normal eyes. OCT in 8 of 13 stage 3 eyes revealed foveal cysts without evidence of cystoid macular edema on fluorescein angiography or biomicroscopy, and 1 lamellar hole. In stage 3 eyes, mild retinal thickening was found in 7 of 9 macular areas (P < .05). OCT commonly reveals foveal cysts in stage 3 idiopathic juxtafoveal telangiectasia. Consistent findings of associated mild macular thickening and lack of late petaloid hyperfluorescence on fluorescein angiography suggest that these cysts differ in pathophysiology from cystoid macular

Use of intravitreal triamcinolone acetonide injection in unilateral idiopathic juxtafoveal telangiectasis

Idiopathic juxtafoveal telangiectasis (IJT) is one of the primary retinal telangiectasia that can result in visual impairment attributed to resultant cystoid macular oedema and rarely, choroidal neovascularization. The authors herein report successful treatment using intravitreal triamcinolone acetonide in a case of unilateral IJT that did not respond to prior laser photocoagulation. Serial optical coherence tomography was used to monitor the effectiveness of intravitreal triamcinolone acetonide in the treatment of IJT.

LONG-TERM JUXTAFOVEAL RETINAL TELANGIECTASIA

To determine the long-term natural history of idiopathic juxtafoveal telangiectasia (IJRT). Record review in 2 university-based and 2 private vitreoretinal practices sought patients with IJRT documented by color photographs and fluorescein angiograms (FAs) during the period January 1, 1980, to December 31, 1993. Patients then had repeated examinations and FAs. Twenty patients with IJRT in 32 eyes had follow-up examinations. Fifteen patients had color photographs and FAs, and one had color photographs alone. Follow-up varied from 10 years to 21 years (average, 15 years). Six eyes were treated by laser photocoagulation at onset. Twenty-four of the 26 untreated eyes lost vision as measured by Snellen testing. Visual loss and morphologic progression depended on IJRT type. Six of 8 untreated eyes with type IA IJRT lost vision by >/ or = 3 lines (Snellen), 4 to 20/70 or worse. Vision loss was caused by progressive telangiectatic changes and intraretinal edema. Fifteen of 20 initially untreated eyes with type IIA IJRT developed either central retinal pigment epithelium membranes or subretinal neovascularization with loss of vision to 20/80 or less. IJRT prognosis depends on type and clinical features. Long-term prognosis for central vision is poor.

Maladie de Coats et télangiectasies primaires ou secondaires

Coats' disease, or primary retinal telangiectasia, is a rare exudative retinopathy, usually unilateral, that most commonly affects male children in the first decade of life. It is probably a congenital disease, non hereditary, without any associated systemic disorders. The disease is characterized by primary telangiectasia and exudates resulting in possible retinal detachment, vitreo-retinal haemorrhages, neovascular glaucoma and ultimate loss of vision. The primary lesion is most likely a localized progressive disorder of the retinal vascularization development. Its exact aetiology remains unclear, but the disease may be caused by somatic mutation in the NDP (Norrie Disease Protein) gene. A subtype of primary retinal telangiectasia, the idiopathic juxtafoveal telangiectasia confined to the macula, constitutes a second clinical form. Secondary telangiectasia and retinal exudates may occur in relation with local or systemic disorders such as retinal vein occlusion, actinic retinopathy or diabetes.

Photodynamic therapy and intravitreal triamcinolone for a subretinal neovascularization in bilateral idiopathic juxtafoveal telangiectasis

To report combined treatment of photodynamic therapy (PDT) with verteporfin and intravitreal triamcinolone acetonide injection for subfoveal neovascularization secondary to bilateral idiopathic juxtafoveal telangiectasis. Interventional case report. A patient with a subfoveal neovascularization secondary to bilateral idiopathic juxtafoveal telangiectasis was treated with PDT plus an intravitreal injection of 4 mg of triamcinolone acetonide. Leakage in the late-phase of fluorescein angiography resolved with attenuation of telangiectatic vessels and improvement in visual acuity from 20/200 to 20/50. At 9 months post-treatment, recurrent leakage was treated with repeat PDT and intravitreal triamcinolone. One year after initial presentation, visual acuity was 20/60 with no leakage on fluorescein. Combined treatment with PDT and intravitreal triamcinolone acetonide resulted in regression of a subfoveal neovascular membrane and improvement in visual acuity during the course of follow-up.